Impaired Cholesterol Synthesis Rate in Fibroblasts and Reduced Cellular Uptake of LDLs Derived from Peroxisome-deficient Patients Cause Cellular Cholesterol Deficiency in Peroxisome-deficient Fibroblasts

Annals of the New York Academy of Sciences

Volumn 804, Issue , 1996, Pages 752-755

  Mandel, Hanna ^a   Getsis, Miriam ^a   Rosenblat, Mira ^b   Berant, Moshe ^a   Aviram, Michael ^b  

^a RAMBAM MEDICAL CENTER   (Israel)

^b TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

Author keywords

[No Author keywords available]

Indexed keywords

LOW DENSITY LIPOPROTEIN;

CASE REPORT; CELL METABOLISM; CHOLESTEROL SYNTHESIS; CONFERENCE PAPER; CONTROLLED STUDY; DISORDERS OF PEROXISOMAL FUNCTIONS; FIBROBLAST; FIBROBLAST CULTURE; HUMAN; HUMAN CELL; PEROXISOME;

EID: 0030469497     PISSN: 00778923     EISSN: None     Source Type: Book Series    
DOI: 10.1111/j.1749-6632.1996.tb18691.x     Document Type: Article

References (8)

1. GOLDSTEIN, J. K., M. S. BROWN, R. G. ANDERSON, D. W. RUSELS & W. J. SCHNEIDER. 1985. Receptor-mediated endocytosis: Concepts emerging from the LDL receptor system. Annu. Rev. Cell Biol. 1: 1-39
2. KRISANS, S. K. 1992. The role of peroxisomes in cholesterol metabolism. Am. J. Resp. Cell Mol. Biol. 7: 358-364.
3. KRISANS, S. K., J. ERICSSON, P. A. EDWARD & G. A. KELLER. 1994. Farnesyl-diphosphate synthase is localized in peroxisomes. J. Biol. Chem. 269: 14165-14169.
4. BIARDI, L., A. SREEDHAR, A. ZOKACI, N. B. VARTAK, B. L. BOZEAT, J. E. SHACKELFORD, G. A. KELLER & S. K. KRISANS. 1994. Mevalonate kinase is predominantly localized in peroxisomes and is defective in patients with peroxisome deficiency disorders. J. Biol. Chem. 269: 1197-1205.
5. HODGE, V. J., S. J. GOULD, S. SUBRAMANI, H. W. MOSER & S. K. KRISANS. 1991. Normal cholesterol synthesis in human cells requires functional peroxisomes. Biochem. Biophys. Res. Commun. 181: 537-541.
6. MANDEL, H., M. BERANT, D. MEIRON, A AIZEN, J. OIKNINE, J. G. BROOK & M. AVIRAM. 1992. Plasma lipoproteins and monocyte-macrophages in a peroxisome-deficient system: Study of a patient with infantile Refsum disease. J. Inher. Metab. Dis. 15: 774-784.
7. AVIRAM, M. 1993. Modified forms of low density lipoprotein and atherosclerosis. Atherosclerosis 98: 1-9.
8. MANDEL, H., M. GETSIS, M. ROSENBLAT, M. BERANT & M. AVIRAM. 1995. Reduced cellular cholesterol content in peroxisome-deficient fibroblasts is associated with impaired uptake of the patient's low density lipoprotein and with reduced cholesterol synthesis. J. Lipid Res. 36: 1385-1391.